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Degenerative Myelopathy (DM)

Canine Degenerative Myelopathy (DM), is a heartbreaking progressive neurological disease affecting the spinal cord that mimics the symptoms of ALS (Amyotrophic lateral sclerosis) and/or PPMS (Primary-progressive multiple sclerosis). It is also known as Chronic Degenerative Radiculomyelopathy (CDRM), and Canine Degenerative Myelopathy. It is currently incurable and fatal.

The Spinal cord contains neurons (nerves) that carry messages between the brain and the rest of the body.  The spinal cord is surrounded by a protective, insulating sheath of tissue, called Myelin.  If a dog has DM, this Myelin Sheath starts to break down leaving the neurons exposed, which then become damaged and can die. This causes a loss of communication between the brain and the body and the messages to function do not get through.  This damage starts with the dog losing the ability to use their rear legs, and will progress up through the core (middle of the dog) and then eventually reach the front legs, before finally affecting vital organs such as the respiratory system. 


This process though doesn't happen suddenly, it is a slow process that takes many months and in some cases even years (from first symptoms to End Stage, the time span can be as much as 3 years or so). How long a dog can survive with DM depends upon many factors.. the age of the dog at onset, the demeanor of the dog and of course the ability of the owner to be able to care for the dog through the DM journey, and that can depend on many factors including personal circumstances, family, work and finances.


DM is an older dog disease with most signs starting to show around the average age of 8 years and older and there is no differentiation between male or female dogs.  It used to be thought of as a GSD or large dog disease, but has now been found in many different breeds of dogs including: German Shepherd Dogs, Boxers, Pembroke Welsh Corgis, Chesapeake Bay Retrievers, Rhodesian Ridgebacks, Bernese Mountain Dogs, Wire Fox Terrier and others.  In 2009, a mutation in the SOD1 gene was discovered to suggest the risk factor of DM, it is however, not a diagnosis of DM.  This mutated gene has now been found in over 124 breeds.  Male & female dogs are affected equally. 

RESEARCH ARTICLES

DNA TEST LABS

What Causes DM ?

The actual cause of this disease is still unknown. Research has shown a mutation in the SOD1 gene is a risk factor for developing Degenerative Myelopathy in many breeds, although this is not a diagnosis of DM. Mutations in SOD1 are also associated with familial Amyotrophic Lateral Sclerosis, ALS, (Lou Gehrig's disease) in people. Additional genes have since been found in: The Bernese Mountain dog, (a second mutation of the SOD1 gene often referred to as SOD1-B or exon 1) and another in the Pembroke Welsh Corgi, (a mutation found in the SP110 gene, known as a Modifier gene) this will reduce the age of onset for DM. 

Though not clinically confirmed, there is strong evidence to suggest some breeds may get a form of the disease very similar to Primary Progressive Multiple Sclerosis (PPMS). In the former DM Flash Test, an Alelle 1101J was implicated in DM of the German Shepherd Dog, a gene associated with PPMS in people.


We have a lot more knowledge about DM than we did when it was first recognised, but sadly at this time, there are still many unknowns about the causes of DM, whether that is additional genes or environmental issues, but it is clear more research is needed to uncover these 'unknowns' and to help us find treatments and finally a Cure.

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How Can I Tell If My Dog Has DM ?

At this time, there is sadly still no definitive test to confirm DM in a living dog.  DM can only be a 'Confirmed Diagnosis' by having a necropsy (autopsy in humans) performed after a dog has passed. 


A series of tests, known as 'Rule Out Testing' is important to have carried out if possible, to rule out the many other diseases and conditions that can have the same or similar symptoms as DM.  If nothing conclusive is found from this testing, you will be left with what is known as a 'Presumptive Diagnosis of Exclusion'.


Some conditions can be treated or require different therapy, for example a dog with DM will require exercise to help slow DM, by means of maintaining muscle mass, whereby some disc issues can require restricted exercise to prevent further damage.

 

CONDITIONS SIMILAR TO DM

Like most diseases, DM has a set pattern of progression, there can be slight variations in...time scales, responses

of the patient to symptoms (due to their individual biological, emotional and physical make-up) and slight variations of some lessor symptoms, when or if they occur.  However, these will not alter the established path of the disease.


DM has a slow onset and is NOT Painful in itself.  

If any of the early symptoms listed below occur suddenly or if your dog is showing any signs of pain, you are most likely not dealing exclusively or at all, with DM.


DM does NOT come on suddenly.

Other disorders such as Disc disease, Disk herniation, Tumors and FCE (a stroke in the spinal cord) can also cause similar symptoms to DM, but with a much more rapid onset.


It is important to try to establish as far as is possible from the start if your dog may have DM.

Other conditions and diseases can look like DM and have the same or similar symptoms, but some can be cured or require different treatment or therapy.  For Example: DM dogs need exercise to help slow DM by maintaining muscle mass.  Some conditions can require restricted exercise to prevent further damage.


Lots of the Symptoms that look like DM, can be other diseases or issues.

Eg. Knuckling of the paws, dragging of the legs, dipping down in the back, difficulty getting up and even appearance of lack of      pain can be caused by other issues that can mimic the symptoms of DM. 


DM is extremely rare in a dog under 5 years of age.

If your dog is younger, it is extremely unlikely it is DM, but another condition or even another 'Myelopathy', but not DM, Which is also known as Canine Degenerative Radiculomyelopathy (CDRM).

STAGES OF DM - Progression

Stage 1 - VERY EARLY/EARLY STAGE

DM initially affects the back legs only, NOT the front legs.

At first you may notice rear limb weakness and slight muscle loss, difficulty transferring from lying down or sitting to standing, and/or an inability or reluctance to climb stairs, jump into the car, or onto furniture.  This may be thought of as aging or Arthritis and can of course, often be a contributing factor.  You may notice a scraping sound on the pavements/ sidewalks as the dog may drag one or both paws when walking and you notice when you check the paws the middle two nails are worn further than the others.  At this point you will most likely have seen your veterinarian and DM could have been mentioned.  Eventually the scraping can become 'knuckling' of the paws (walking on the tops of one or both paws - this is loss of conscious proprioception)

NOTE: The symptoms above can also be typical of other diseases such as Arthritis, Hip Dysplacia, Spinal issues such as IVDD and other conditions. MAKE SURE that your veterinarian, or a veterinary neurologist, examines your dog for other conditions that may also involve the spine.


Stage 2 - INTERMEDIATE STAGE

Worsening signs of weakness in the rear end of the dog. 

As you enter Stage 2 Intermediate Stages, there will be a worsening of Stage 1 symptoms.  The tail may become limp.  There will be more crossing of the hind limbs, getting tangled as walk like a drunken sailor. They can get stuck or collapse as they turn as their legs won't go where they want them to.  They will 'slink' down in their back end as their legs become unable to support their weight fully.  Fecal and/or Urinary incontinence can occur.  As they progress through the Intermediate stage, they can become anxious and vocalization can increase.  Twitching of the rear limbs may become more 'violent' when laying down or even asleep, due to misfiring of the nerves.  You may note a hoarseness in the bark and/or loss of volume.  They will eventually become unable to stand up at all unaided using their back legs and will start pulling themselves around using their front legs, this is known as being 'Down in the Back'. Pressure sores can develop as they are unable to fully move around so much. UTI's can develop.  They may start to have what appears to be weakness in the front legs, this is often not progression of DM though, but 'compensation pain' as a result of additional stress from pulling themselves around by their front half and can mostly be managed by mild pain meds and massage.

NOTE:  A dog with the right aids, support and care can manage for quite a long while at this stage, although as DM progresses, DM will speed up slightly.


Stage 3 - LATE STAGE

Movement of weakness through the core of the dogs body (middle section) to affect the front end and legs.

At this stage, progression can be more rapid, but can still plateau for quite a while.  You will see floppiness in the core, and they will now become unable to use their front legs and be unable to sit up.  There will be more muscle loss in the shoulder area as well as front legs and hind quarters. UTI's can become more difficult to overcome and pressure sores may be more persistent.  They will progress to being unable to keep themselves in an upright position on their belly and will need assistance to do this.  The nervous system's spinal cord and brain stem are the only structures actually affected by DM, however, weakness from DM can have secondary effects on the dog such as the pressure sores and systemic infections.

NOTE:  A dog can pass naturally from End Stage DM, however vigilant monitoring and 24 hr care is essential at all times to ensure a crisis does not arise and there is no distress for the dog. in which case most owners would ensure euthanasia is carried out to prevent this happening.


Stage 4 - VERY END STAGE

Progression to Multi Organ Failure.

The disease will progress to the brain stem and eventually to the cranial nerves which may affect vital organs such as the diaphragm and breathing.  There can eventually be kidney, lung and heart failure.  Asphyxiation can occur.  Death from DM, should it get to that stage results from multi organ failure. 

NOTE:  Very End Stage can be very distressing for both dog and owner.

Most people who care for their dogs even through End Stage DM, monitor their dogs vigilantly and choose euthanasia as soon as changes occur to ensure a crisis situation of Very End Stage is not reached. 

The U.S based Orthopedic Foundation for Animals are able to offer a DNA saliva test to screen for the mutated gene that has been seen in dogs with Degenerative Myelopathy. The test is only recommended for predisposed breeds, but can be performed on DNA from any dog on samples collected through swabbing the inside of the animal's cheek with a sterile cotton swab or through venipuncture.

The DM DNA test is now available from many Testing labs around the world.

The results reported are:

•   Normal / Normal (N/N, or 'clear'): The dog does not have the mutation and is extremely unlikely to develop Degenerative Myelopathy. There have been cases in which dogs that tested clear were found to have DM upon necropsy. This information was given to Dr Keller from the OFA. Dr Coates performed necropsy. It is important to note the OFA statement on their website that states "Recent evidence suggest that there are other causes of DM in some breeds".
•   Normal / Abnormal (N/A or 'carrier'): The dog has one mutated copy of the gene (is heterozygous) and is a carrier but will not have degenerative myelopathy though there has now been several cases of Carriers developing DM. It will be possible for it to pass the mutation to offspring. A thorough examination of the dog's pedigree and DNA testing should be undertaken prior to breeding a dog with this result.
•   Abnormal / Abnormal (A/A or 'at risk/ affected'): The dog has two copies (is homozygous) for the mutation and is at risk for Degenerative Myelopathy.

There was a former test known as the DM Flash Test which looked for a change in Allele 1101J. Due to lack of funding the Flash Test is no longer available. It was designed specifically for the German Shepherd breed. Unless more research exists, the Flash Test may never exist again.

​GENETICS

Breeding risks for Degenerative Myelopathy can be calculated using the Punnett Square:

  • If both parents are clear (N/N) then all of the puppies will be clear

  • If one parent is a carrier (N/A) and one is clear (N/N) then roughly 50% of the puppies will be clear and 50% will be carriers
  • If both parents are carriers (N/A) then roughly 25% will be clear (N/N), 50% will be carriers (N/A), and 25% will be affected (A/A)
  • If one parent is clear (N/N) and one parent is affected (A/A) then all puppies will be carriers (N/A)
  • If one parent is a carrier (N/A) and one is affected (A/A) then roughly 50% of the puppies will be carriers (N/A) and 50% will be affected (A/A)
  • If both parents are affected (A/A) then all puppies will be affected (A/A)

STATISTIC

Breed Registry

Rank

Evaluations

Percent

Abnormal

Percent

Normal

Pembroke Welsh Corgi

1

1756

52.4

10.0

Hybrid Dog ***

2

148

50.0

39.9

 Boxer

3

2099

45.5

16.7

 Hovawart

4

50

24.0

50.0

 Collie

5

51

23.5

43.1

German Shepherd Dog

6

2784

16.9

50.8

Cardigan Welsh Corgi

7

466

12.4

52.1

 Kerry Blue Terrier

8

311

12.2

47.9

Bernese Mountain Dog

9

1813

12.0

40.3

 Chesapeake Bay Retriever

10

1735

10.2

44.8

Pug

11

131

7.6

64.1

 Bloodhound

12

177

6.8

49.7

Rhodesian Ridgeback

13

2241

6.7

54.0

 Puli

14

105

5.7

68.5

 Canaan

15

55

5.5

61.8

Labrador Retriever

16

124

4.0

91.1

Borzoi

17

690

2.2

71.9

Mastiff

18

51

2.0

76.5

Shiloh Shepherd

19

184

1.6

72.8

Golden Retriever

20

91

1.1

97.8

 Poodle

21

389

0.5

87.7

*** A hybrid dog is not a BREED; it is a hybrid, which means it is a mix of more than one purebred dog.

also know as Designer Dogs or Boutique Dogs

Statistics from the Orthopedic Foundation for Animals. Evaluations through December 2012

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TREATMENTS

Degenerative Myelopathy is an irreversible, progressive disease that cannot be cured. There are no treatments that have been clearly shown to stop or slow progression of DM though Aminocaproic Acid and NAC have been used with some success to slow down the progression of the disease when started early enough into the disease. Evidence is anecdotal, but some have reported a slowing of progression and a rare few have gone into a type of remission with the use of those two medications along with vitamin supplementation and homemade diets. Speak with your vet about what course to take in treating your dog. The protocol can be found in the helpful links section under Publications. The protocol was designed for the form of DM that most often strikes the German Shepherd Dog.

Exercise

Exercise has been recommended to maintain the dog's ability to walk. Physiotherapy may prolong the length of time that the dog remains mobile and increase survival time.

Canine hydrotherapy (swimming) may be more useful than walking.

Use of a belly sling or hand-held harness allows the handler the ability to support the dog's hind legs for exercising or going up and down stairs. A supportive harness that cradles the entire pelvis is recommended. "towel walking" can put pressure on the bladder and internal organs.

A 2-wheel dog cart, or "dog wheelchair" can allow the dog to remain active and maintain its quality of life once signs of weakness or paralysis of the hind limbs is detected.

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